Glioblastoma with concomitant moyamoya vasculopathy in neurofibromatosis type 1: illustrative case

In a case of concurrent glioblastoma and moyamoya vasculopathy, it is arduous to safely perform surgery because the brain is highly vulnerable and collaterals are sometimes well developed. In addition, radiotherapy carries a risk of aggravating moyamoya vasculopathy, and chemotherapeutic agents also have a risk of interfering with collateral development.

A 48-year-old woman with neurofibromatosis type 1 was admitted because of left hemiparesis and hemispatial neglect. Brain imaging studies revealed a large mass with peripheral enhancement in the right frontal lobe and occlusion of the bilateral middle cerebral arteries with an abnormal vascular network at the base of the brain. Total tumor resection was performed, and the pathological diagnosis was isocitrate dehydrogenase-mutant glioblastoma. Radiotherapy with a total dose of 60 Gy was delivered with concurrent temozolomide, and thereafter six cycles of adjuvant temozolomide were given. Progression of moyamoya vasculopathy without symptoms was observed after the completion of each of radiotherapy and adjuvant temozolomide.

The authors present the first adult case of glioblastoma with moyamoya vasculopathy. Careful consideration and attention should be given throughout treatment to avoiding moyamoya vasculopathy-related ischemic and hemorrhagic events. Although the patient did not exhibit neurological deterioration, progression of moyamoya vasculopathy occurred early after radiotherapy and continued thereafter.